American Board of Internal Medicine (ABIM) Certification Practice Exam

What should a diagnosis of hepatic vein thrombosis suggest?

1. Multiple myeloma

2. Leukemia

3. Polycythemia Vera

4. Chronic renal failure

The correct answer is: Polycythemia Vera

A diagnosis of hepatic vein thrombosis, also known as Budd-Chiari syndrome, should suggest the presence of conditions that lead to thrombophilia or hypercoagulability. Polycythemia vera is a myeloproliferative neoplasm characterized by an increase in red blood cells, which can lead to increased blood viscosity and a higher risk of thrombosis. The mechanism behind this association involves the overproduction of red blood cells, which raises the likelihood of clot formation in the hepatic veins due to stasis and impaired blood flow. Patients with polycythemia vera may also have associated mutations, such as the JAK2 V617F mutation, that contribute to a hypercoagulable state. In contrast, while other options like multiple myeloma, leukemia, and chronic renal failure can also have thrombotic complications, they are not primarily associated with hepatic vein thrombosis in the same way as polycythemia vera. Thus, in the context of hepatic vein thrombosis, polycythemia vera is the most relevant condition to consider as it directly contributes to the pathophysiology behind the thrombotic event.